News from Dysautonomia International

This video by Dysautonomia International gives a basic overview of POTS (Postural Orthostatic Tachycardia Syndrome). The video is on Facebook but you don't have to have an account to view it.

Patients with POTS or other types of Orthostatic Intolerance may not find much useful information here but it might be helpful for friends and family members who know nothing about POTS.

Code:

https://www.facebook.com/DysautonomiaInternational/videos/1079102622188616/

Note that this video has very general information on POTS. Some of the information may not apply to ME patients (e.g., exercise suggestions). I still think it's a helpful overview but I wanted to warn folks so that they would not forward it to folks who might not understand these issues.

 

Not had a chance to watch this, so can't vouch for it being of interest but thought it might have potential.

 

I am not sure if this is the same video that was posted on PR (along with a second video) b/c I have not had a chance to watch it yet. But I am definitely planning to do so. Thank you for posting it here and I am looking forward to watching it later today.

 

@Andy I just watched this video which was PHENOMENAL and thank you for posting it. I took notes and am posting a summary of the video (combined with my own commentary ) in the hope that it will be helpful for others. I plan to watch the two videos on PR (not sure if one of them is this one yet?) and will post something over there, too. I am always amazed at the quality of videos from "Dysautonomia International" and this speaker, Dr. Brent Goodman, from Mayo Clinic, was so impressive and on target, that it is baffling to me why Mayo in general is so sucky and does not take any of these conditions seriously? There is such a dichotomy between the medical knowledge, testing, and treatment of this doctor vs. Mayo which throws everyone under the "Central Sensitization/Psychiatric" bus.

In summary, he spoke about the connections between Autoimmune POTS, Dysautonomia, and Mast Cell Activation Syndrome (MCAS) and he could have been describing my life history over the last 4+ years. He said that 73% of cases of POTS started out with a virus or pathogen (and in his example it was a stomach bug but in my case it was mono and then a second respiratory infection). He said the first cases of known POTS were with soldiers in 1871 but the actual term & diagnosis was not developed until 1993! WTF took them so long? And even since 1993, most doctors still do not know about POTS from my experience.

He talked about "Acute Autoimmune Autonomic Neuropathy" and how with this autoimmune pathogenesis the treatment is IVIG. No wonder why IVIG has been so incredibly helpful for me. He said that POTS often causes "autonomic system remodeling" which can become chronic, persistent and refractory (which was my case prior to IVIG & Rituximab) and he described the shortness of breath that I experienced when walking without wheelchair (for 3+ years prior to my treatment). Today it is 5:00 pm and I have not yet needed to use the wheelchair inside of my apt. Today I had a woman come to my apt to cut & color my hair (which I've been doing since 2015) and she had never seen me walk without the wheelchair, and like everyone else, she was in awe. I was walking at a fast pace from room to room while carrying things, and was not short of breath, and she was amazed. I am also no longer allergic to any products or smells (from the MCAS piece of my illness).

The video said how some people can heal from POTS with just life-style changes and meds like Midodrine but this was not the case for me. I continue to use all kinds of life style changes, and still take Midodrine, but prior to IVIG and Ritux, they were not enough to walk without wheelchair, or to stand for more than 30-60 seconds without gasping for air. And now I can do four loads of laundry, stand and wash dishes for 30 min, etc. I am wondering how far I can really walk now, and if I can climb stairs or drive now, but have not had the opportunity to try yet and am still trying to be careful and not push too far, even though it is incredibly tempting.

In the video, the doctor said how Neuros do not understand these conditions and said that the Neuros need to "Put on their big boy and big girl pants" which made me laugh b/c if I was still waiting for one of the three Neuros that I saw to help me, I think I would probably be dead vs. posting here today. He talked about how the anti-muscarinic auto-antibodies are high in a percentage of POTS patients (I did not catch the number) and they are high for me, along with the beta-adrenergic auto-antibodies, which I don't think he mentioned unless I missed it. These auto-antibodies (along with others) are what led my main doctor to diagnose me with "Autoimmune POTS" (vs. a different sub-type of POTS) and this video completely concurs with this.

Lastly, he spoke about Mast Cell Activation Syndrome (MCAS) and how mast cells are everywhere, yet they can also be "regional" to a specific system in the body. So one person with MCAS could have predominantly GI issues, but another could have skin issues, or breathing issues, etc. I already knew this from my MCAS specialist but am not sure how common this info is (and many doctors have never heard of MCAS or do not believe it is real). He said that 1/3 of POTS patients will develop MCAS and this is exactly what happened to me. I developed POTS in Jan 2013 and I developed MCAS in March 2015.

He said that MCAS has important implications for the immune system because mast cell degranulation can trigger autonomic dysfunction/instability AND the converse, that autonomic dysfunction can trigger MCAS is also true. He said he starts his MCAS patients on: Cromolyn, Allegra & Zantac and I was started on these meds in 2015 but they did absolutely zero to stop the progression of my anaphylaxis & allergic reactions. The winning combo for me was Ketotefin, Zyrtec, Pepcid, some supplements like Quercetin, and Atarax (vs. Benadryl) as my rescue med. But even with that combo, and being able to tolerate many normal foods again, the reactions continued and did not go into remission until I began IVIG. I thought it was a fluke but the MCAS remission has continued for 16 months. Now what happens when I eventually stop IVIG, I do not know? The IVIG alone put my MCAS into remission, a full year before I did Rituximab, so Ritux played no role in that process. But it was not until after Ritux that I regained normal muscle strength and the ability to stand for as long as I want (longest is now two hours) and the ability to walk short distances without wheelchair.

In the video, he said that "aggressive pharmacotherapy" is necessary for MCAS, which I one million percent agree with, and saved my life. He also said that MCAS (unless it is Mastocytosis which is a primary disease) is secondary and is not the primary cause vs. an end-product. My MCAS specialist said exactly the same thing and this reinforced what I had been told which was cool.

This video made me think that there is a viral/pathogen triggered illness, that truly looks like ME/CFS in the early stages, but then shifts into auto-immunity, POTS, and MCAS. He described my illness but did not give it a name. I was explaining to the woman who cut my hair today (b/c she was asking questions re: my recovery and I did not mind) that I do not feel fatigue or lack of energy vs. that my muscles had been extremely weak and I literally could not control my HR, BP, or ability to breathe when I stood up without wheelchair and now I can! And in addition, the allergic reactions are gone.

The POTS is not actually gone, and I do not want to misrepresent this. The POTS related symptoms have been the most intractable but they are vastly better and the only explanation is that the POTS was Autoimmune and now with less auto-antibodies from IVIG and Ritux, my overall dysautonomia is better. I have additional auto-antibodies not mentioned in the video that we believe contributed to my muscle/breathing weakness but in the video, he described the overall auto-immune profile which I completely fit. Except I have autoantibodies attacking my thyroid, calcium channels, GAD65, etc, vs. he was talking about Celiac, Sjogrens, Lupus, RA, and auto-antibodies that I do not have.

Lastly, in the video, the doctor mentioned IVIG and Plasmapheresis (PP) as the main treatments but in my experience, no doctor in the US was willing to prescribe PP for me so I never had the opportunity to try it. If it worked, it would have given quick relief which would have been diagnostic, but the results are also very fleeting. Versus Rituximab (for someone in my case) is much longer lasting and if you are a responder, in the absolute ideal dream scenario, the new B-cells can grow back healthy, and auto-antibody free, and lead to a lasting remission (but Rituximab was not mentioned in this video and I am adding that as a side note). I am going to Google this doctor and am really looking forward to watching the other videos on PR. Thank you so much @Andy for posting this, it was so cool to watch!

 

Hi @Gingergrrl,

Good summary.

Did you watch the other video too? I think it may be even more relevant to you.

Its obviously only one study but I found the distribution of antibodies in autoimmune dysautonomia phenotype to be quite surprising!



I believe the Autoimmune dysautonomia panel includes the beta adrenergic & muscarinic antibodies that are usually focused on in this field - interestingly quite a low percentage!

I checked myself for antiphospholipid antibodies previously as I have a very strong family history of migraine, miscarriage, multiple DVTs, PEs, vascular issues etc. Thankfully it was negative and the clotting issue may just be due to Factor V Leiden mutation which we all have confirmed.

Very interesting to see such a large percentage of Early Sjogren's antibodies too. My mother is awaiting lip biopsy investigation for Sjogren's currently and her specialist remarked that he sees a lot of POTS patients in his clinic.

Ryan

 

Glad it was of use @Gingergrrl and thanks for your write up of it.

 

Thanks, Ryan!

I didn't (yet) and I haven't had a chance to go back to that thread on PR and see if the two videos linked there by KMS1990 are the same as the ones here (since DI makes so many amazing videos and lecture series). I am hoping to be able to do this later today.

I was also checked for antiphospholipid antibodies in 2016 and was negative. I was checked b/c we wanted to make sure I was not a blood clot risk prior to starting high dose IVIG.

 

I just watched the second video (posted by Ryan) and can now confirm that these are the same two videos posted on PR. This video of Dr. Jill Schofield was absolutely amazing and again, she could be describing my history of the past 4+ years. I took detailed notes and am posting a summary of the video in case it is helpful for anyone.

This second video is called "IVIG treatment in refractory autoimmune dysautonomia" and made me realize that "Refractory Autoimmune Dysautonomia" could be the title of my illness (plus MCAS). Ironically, when I did the Cell Trend testing in 2016, I asked the head of Cell Trend Labs, Dr. Harald Heidecke, if he knew of a doctor in the US who could help me interpret the results and get treatment and the name that he gave me was Dr. Jill Schofield from this video!

I researched her on-line w/the location he gave me (University of Colorado) but she was no longer there and I was unable to track down any contact info for her at that time. This was over a year ago, and I have not tried again, but if anyone knows how to contact her, I would love the info! Although my own doctor's treatment has literally matched her recommendations in the video so I am not sure that anything would have been done differently (which was very comforting to me).

She divided the auto-antibodies into two circles (purple & red) and I wish I knew how to copy a slide from a video to post here but I don't know how. The red circle had the common autoimmune antibodies but the purple circle had the more rare ones which I have. From the purple circle, I have the CA+ Channel Ab, GAD65, anti-muscarinic, and beta-adrenergic Abs (I have four)! She noted how the anti-muscarinic and beta-adrenergic tests are not available in the United States and I still find this baffling that the US is so behind. I am so grateful that I was able to do these tests from Germany b/c they helped my doctor and helped w/the insurance.

Then she talked about how she uses IVIG for autoimmunity and Autoimmune POTS (which I was diagnosed with). She said that many patients have to use IVIG indefinitely, or go on to replace it with something else, and she used Rituximab as an example. She was so nuanced on this issue that she noted how if you go from IVIG to Ritux and do the required Hep B testing, some patients will show false immunity for "Hep B Core antibodies" b/c of passive, temporary immunity from IVIG which is EXACTLY what happened to me. I've never had Hepatitis, and never been vaccinated for it, but had passive autoantibody transfer from IVIG. Both my main doc and MCAS doc (who is an immunologist) confirmed this is what happened, based on my testing pre-IVIG which was negative, and on the half-life of IVIG. But my main doc is unbelievably thorough and did additional testing to be 100% sure prior to Ritux.

Dr. Schofield discussed that IVIG is expensive and can be burdensome to administer (which is true) and that it is safest to do a very slow infusion speed to avoid aseptic meningitis (which is exactly what I do). She discussed pre-meds and mine are similar but different (mine are Atarax, Tylenol and Pepcid). She said that many docs or infusion centers don't know that they should never run concurrent/piggy-back IV fluids with the IVIG b/c this can mess up the efficacy of the IVIG. Luckily, I have NEVER had concurrent fluids, b/c I do better with less total fluid volume, so I did this right!!!

She discussed how if a previously healthy person develops POTS after an infection, surgery, etc, this is suspicious for autoimmunity as the cause and this is exactly what happened in my case. She said she does not use IVIG for all of her POTS patients but for those with positive auto-antibodies who become so sick that they can no longer work (like me), these are the best candidates. She does a skin biopsy which she said can help with insurance approval but I did not do this.

She said that 82% of her cases are female and the average age in their 30's but I was 42 when the POTS began. She said that she has had patients who were sick for over 20 years, with no improvements from any treatment, until they treated the underlying cause with IVIG. And even some of these patients who were sick for 20+ years got better with IVIG (which happened in my case, except I will be sick for 5 yrs in Jan 2018 and not 20 yrs).

She said that many of her patients have a positive QSART test for autonomic neuropathy/abnormal sweat response and I was abnormal on this test when I did it at Stanford (even though they never interpreted it for me or what to do for it). She said there is no "consistent autoimmune fingerprint" but that 80% of her patients had POTS and one of her charts showed an "Autoimmune Phenotype" which I matched on several points like having a positive ANA titer of 1:160, Thyroid auto-antibodies, etc.

She said that Mayo Clinic's "Autoimmune Dysautonomia Panel" was actually ruling people out which it shouldn't have which was very interesting to me. I've never done this panel, and did the PAVAL Panel instead. She did not mention the PAVAL Panel, but I am now feeling that it was a better choice, b/c it was critical in getting my treatment approved.

She said that treatment with IVIG is usually for a minimum of one year and that a full response often takes 1-2 yrs. I have now done IVIG for 16 months, with the last 11 months being high dose. My last approved IVIG cycle is in Dec (in about 2 wks) and I will be speaking with my doctor later in Dec to figure out the plan for next year. I will tell him all about Dr. Schofield's presentation and I actually just e-mailed him the link to the video in case he had not seen it. Dr. Schofield had POTS patients who had used a wheelchair for years, but after IVIG no longer had to use wheelchair, which gave me immense hope! I wish she would have discussed Rituximab in more detail, but other than one brief mention at beginning of video, she did not.

Thanks again to Ryan and Andy for posting these videos, they were amazing!

Edit: typos and clarity

 

I often skip over this "other health" forum [limited energy] so I would have missed this thread if not for a mention of it in another forum.

I have not watched the videos yet but thanks @Andy for posting them. I'll come back and read the comments after I've had a chance to watch them (which may be a while).

 

@ahimsa On both boards, I only click "new posts" so I see everything and never pay attention to what forum it is in. But I guess others actually check each forum by hand?

 

Yes, I usually click New Posts to start with.

But often I will go to individual forums that are less interesting to me and click "Mark Forums Read" for just that forum. It makes the list of new posts shorter and easier to skim.

I'm not very consistent in how I read the forums, though

 

I think you will really like the videos and I hope my summaries are helpful and not too much to read!

There is no right or wrong way and I click new posts b/c don't want to miss anything... except for the cat thread

 

Be careful... or I might start sending you a bunch of kitty photos via private message.

More seriously, I'm looking forward to watching the videos but need to plan it for when I have some brainpower and won't be interrupted.

 

Hi @Gingergrrl,

I took a screengrab of the page you referred to, it is useful to see the neuronal & systemic categories.



Ryan

 

Ryan, How do you take a "screengrab" from a video to post the slide in your message? I have an iMac computer in case this is relevant and would love to learn how to do this! Thank you for posting that slide and it was the exact one that I was referring to in my summary and had wanted to share. I have four things (the Voltage gated Ca channel Ab, GAD65, and the muscarinic and adrenergic receptor Abs) from the purple circle from that slide. Even though I already knew that, it was strange to see it in writing and wish I knew what it all meant.

 

I use the screen clipping tool that is part of Microsoft Office OneNote - not sure if you have that or if it provides same functionality on Mac. This might help though: https://support.apple.com/en-gb/HT201361

Ryan

 

I just made a screenshot of the how to
For a video, better pause it first of course.

 

Thank you Ryan and Marc for the info above and I will try it in the future and see how it goes!

 

Just watched the first one - thanks! I despair at how to get the correct testing and treatment in the UK. My mast cell keeps getting worse and no idea what to do/how to treat it all. So frustrated with having to chase down individual people for each piece of the puzzle who then doesnt connect the other pieces. I love to see a talk like this which connects the dots somewhat.

 

I was just asked about MCAS by someone in the UK with EDS/PoTS, and how it is best to start any investigation of possible mast cell issues. Is there any basic/widely recognised test that they could get on the NHS? Should I advise them to try to find a specialist?

That doesn't sound good!

I found this: http://www.potsuk.org/mcas

I'm not really sure what 2 means, or how it would be tested.